Maruyama T, Kondo K, Tabata K, Yanagisawa N
Department of Neurology, Kakeyu Rehabilitation Center.
Rinsho Shinkeigaku. 1992 Nov;32(11):1294-8.
A 61-year-old civil engineer began to have slowly progressive muscle atrophy in the right shoulder and the left arm at 56 years of age. Muscle wasting became manifest in the left thigh at 59 years and in the right thigh at 60 years. He had mild difficulty in climbing and descending stairs. On examination, although he had notable muscle atrophy in the right trapezius and proximal muscles in the upper and lower extremities, his muscle strength was relatively well preserved. The muscle atrophy was asymmetrical; the right periscapular region and the left upper and lower extremities were more markedly atrophic. In addition, multiple foci of the striking muscle atrophy were noted in the upper trunk and the proximal limb muscles. Fasciculation was not present. Deep tendon reflexes were normal with no pathologic reflexes. Except for a moderately elevated serum creatine kinase level of 709 Ul/l (normal 40-170) and mildly elevated serum myoglobin level of 100 ng/ml (normal < 60), no laboratory tests showed abnormal values suggesting an inflammatory process. Motor and sensory nerve conduction velocities were within normal limits. Electromyography disclosed myopathic and neuropathic changes. Computed tomography (CT) of skeletal muscles showed asymmetrical muscle atrophy and patchy low-density foci. In biopsied left quadriceps and right gastrocnemius muscles which showed partially low density on CT, there was marked variation in muscle fiber size, with necrotic and regenerating fibers, an increased number of centrally placed nuclei, and interstitial fibrosis. There were numerous foci of mononuclear inflammatory cellular infiltration, especially around the blood vessels.(ABSTRACT TRUNCATED AT 250 WORDS)
一名61岁的土木工程师在56岁时开始出现右肩和左臂缓慢进行性肌肉萎缩。59岁时左大腿出现肌肉萎缩,60岁时右大腿出现肌肉萎缩。他上下楼梯时有轻度困难。检查时,尽管他右斜方肌以及上下肢近端肌肉有明显的肌肉萎缩,但肌肉力量相对保留良好。肌肉萎缩不对称;右肩胛周围区域以及左上肢和下肢萎缩更明显。此外,在上躯干和近端肢体肌肉中发现多个明显的肌肉萎缩灶。未出现肌束震颤。深腱反射正常,无病理反射。除血清肌酸激酶水平中度升高至709 Ul/l(正常40 - 170)和血清肌红蛋白水平轻度升高至100 ng/ml(正常<60)外,没有实验室检查显示提示炎症过程的异常值。运动和感觉神经传导速度在正常范围内。肌电图显示有肌病性和神经病性改变。骨骼肌计算机断层扫描(CT)显示不对称性肌肉萎缩和斑片状低密度灶。在CT上显示部分低密度的活检左股四头肌和右腓肠肌中,肌纤维大小有明显差异,有坏死和再生纤维,中央核数量增加,以及间质纤维化。有大量单核炎性细胞浸润灶,尤其是在血管周围。(摘要截断于250字)